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Table 2: Clinical and morphologic features of previously published adults > 40 years of age with unoperated tetralogy of fallot and studied at
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necropsy
Case First
Author
1 White 1929 1 59M 58 58 1 0 4 110/80 + + 100%a— 0 0.12 0 0 + 0 0 450 + 0
2 Volini 1938 2 41M 41 35 35 0 3 170/100 + + 100%
3 Strandell 1939 3 56F 56
4 Feigin
5 43 F Birth 25 18 0 4 230/160 + + 98%
6 Middleton 1947 5 45M 3 44 1 0 4 — + + 21 61 0 0.32 0 + 0 0 0 790 0 +
7 Lian 1949 6 55F Infancy — — 0 3 140/85 + 0 — — 0 — + 0 + 0 — — + +
8 Civen 1950 7 47M 27 — 0 — — 140/80 — — — — — — — 0 0 +
9 Baguena 1951 8 44M 44
10 Miller 1952 9 56M Childhood — 0 0 1 120/85 + + 23 — +
11 Bain 1954 10 69M 1 67 2 0 4 130/75 + 0 13 — 0 — + + 0 0 0 450 + 0
12 Bedford 1956 11 53F 32 48 4 — 3 — + — 126%
13 Rosenthal 1956 12 53F 53
14 Marquis 1956 13 64 F 9 — 0 0 3 150/105
15 47M 4 — 0 0 1 130/100 + + 23 — 0 0.12 0 0 0 +
16 Abraham 1961 14 48F 19 — — 0 4 140/90 + + — — 0 — 0 0 0 +
17 Bowie 1961 15 68F Childhood — — 0 4 140/80 + + 18 56 0 0.24 + 0 + 0 0 512 0 +
18 Meindok 1964 16 62M 57 62 O’ + 1 170/100 + 0 18 — 0 — + 0 0 +
19 Oakley 1966 17 58M 45 53 5 + 4 115/85 + + 19 62 + 0.08
20 Thomas 1987 18 77M 77
a
Hemoglobin measurement as a percentage of a standard based on the method used; bchronic glomerulonephritis; cdiagnosis made at necropsy; dtuberculosis; epatient weighed 48 kg; fmeasured before onset
of atrial brillation;
myocardial infarction secondary to atherosclerotic coronary artery disease;
AF=atrial brillation; BP=blood pressure; C=cyanosis; CHF=congestive heart failure; CVA=cerebrovascular accident; DC=digital clubbing; F=female; FC=functional class; Hct=hematocrit; Hgb=hemo-
globin; HW=heart weight; Hx=history; IE=infective endocarditis; M=male; MVP=mitral valve prolapse; NYHA=New York Heart Association; s/d=peak systole/end diastole; PV=pulmonic valve;
Ref.=reference; RV=right ventricle; VA=ventricular arrhythmias.
Year of
Publication
Ref. Age (yr)
Death&
1942 4 53M 37 51 2 0 4 140/70 + + 98%a— + 0.24f+ + 0 0 0 970 + 0
g
cancer; hsudden death in hospital unassociated with CHF; icomplication of bowel surgery; jpulmonary infarct; kpneumonia; lCHF developed with acute myocardial infarction; macute
at
Sex
Age (yr) at
Diagnosis
c
c
c
c
Age
Duration
(yr) at
Onset
of CHF
(yr) of
CHF
— — 0 4 140/70 + + 18 — 0 0.17 — 0 0 +
32 12 0 2 125/65 + + 120%a— 0 — — 0 0 +h— — 0 +
— 0 0 1 170/90 0 0 — — 0 — 0 0 0 +g— — + 0
73 0 + 1 — + + —n—n0 — 0 + 0 0 0 — + 0
n
IE
NYHA
by
Hx
(1–4 +)
“not polycythemic.”
FC
BP
C DC Highest AF PR
(s/d)
Hgb
Hct
(g/dl)
(%)
a
— 0 0.28 0 0 0 +
a
— 0 — 0 + 0 0 0 600 + 0
i
a
— — — — 0 + 0 — 480 0 +
+ + 23 — 0 — — 0 + 0 — — + +
VA Mode of Death MVP HW
Interval
(sec)
CHF C VA Other RV PV
b
0 750 + 0
d
0 370e0 +
g
0 650 + 0
0.16f0 0 0 + 0 425 0 +
j
0 — + 0
k
0 450 0 +
m
f
+ + 0 0 0 660 + 0
— 710 0 +
(g)
Obstruction
Site of
Outow
Case 968 prolonged surVIVal (74years) In unoperated tetralogy oF Fallot
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REFERENCES
1. White PD, Sprague HB. The tetralogy of Fallot. Report of a case in a noted musi-
cian who lived to his sixtieth year. JAMA 1929;92:787–791.
2. Volini IF, Flaxman B. Tetralogy of Fallot. Report of a case in a man who lived to
his forty-rst year. JAMA 1938;111:2000–2003.
3. Strandell B. Fallot’s tetrad—fall av sällsynt duration. Svenska Läkartidningen
1939;36:1513–1520.
4. Feigin I, Rosenthal J. The tetralogy of Fallot. Am Heart J 1943;26:302–312.
5. Middleton WS, Ritchie G. The tetralogy of Fallot. An account of a patient with
this condition surviving over forty-ve years. Am Heart J 1947;33:250–253.
6. Lian C, Fleury J. Survie jusqú á 55 ans d’une maladie bleue (type Fallot). Arch Mal
Coeur 1949;42:1209–1210.
7. Civin WH, Edwards JE. Pathology of the pulmonary vascular tree. I. Acompari-
son of the intrapulmonary arteries in Eisenmenger complex and in stenosis of the
ostium infundibuli associated with biventricular origin of the aorta. Circulation
1950;2:545–551.
8. Báguena R, Tormo V. Coexistencia de tetralogía de Fallot y persistencia
del conducto arterioso en una persona de cuarenta y cuatro años. Med Esp
1951;26:134–136.
9. Miller SI. Tetralogy of Fallot: Report of a case that survived to his fty-sev-
enth year and died following surgical relief of gall-stone ileus. Ann intern Med
1952;36:901–910.
10.
Bain GO. Tetralogy of Fallot: Survival to seventieth year. Report of a case. Arch
Pathol 1954;58:176–179.
11.
Bedford DE. Two cases of Fallot’s tetralogy, shown at the section in 1929, exhibit-
ing unusual longevity. Proc R Soc Med 1956;46:314–315.
12.
Rosenthal L. Longevity and the tetralogy of Fallot. Br Med J 1956;1:1107.
13.
Marquis RM. Longevity and the early history of the tetralogy of Fallot. Br Med J
1956;1:819–822.
14.
Abraham AS, Atkinson M, Mitchell WM. Fallot’s tetralogy with some features of
Marfan’s syndrome and survival to 58years. Br Heart J 1961;23:110–112.
15.
Bowie EA. Longevity in tetralogy and trilogy of Fallot. Discussion of cases in
patients surviving 40years and presentation of two further cases. Am Heart J
1961;62:125–132.
16.
Meindok H. Longevity in the tetralogy of Fallot. Thorax 1964;19:12–14.
17.
Oakley C, Olsen E. A case of long survival with Fallot’s tetralogy. Br Med J
1966;2:748–753.
18.
Thomas SHL, Bass P, Pambakian H, Marigold JH. Cyanotic tetralogy of Fallot in
a 77year old man. Postgrad Med J 1987;63:361–362.
263
CASE REPORTS IN CARDIOLOGY
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Case 983 Coronary Ostial Dimple (In the
Posterior Aortic Sinus) in the Absence of
Other Coronary Arterial Abnormalities
Jamshid Shirani, MD and William C. Roberts, MD
Normally 2 coronary arteries arise from the aorta, 1 from the aortic wall enclosing
the right aortic sinus and the other from the aortic wall enclosing the left aortic
sinus; the wall enclosing the posterior, i.e., noncoronary sinus, is smooth and devoid
of any “dimples” or “buds” or other suggestions of a residua of a potential coronary
ostium. Recently, we examined a heart with 2 normally arising coronary ostia and a
dimple of an undeveloped coronary ostium in the third aortic sinus. Such an occurrence has not been seen by us in approximately 10,000 other hearts examined in a
similar manner. This report briey describes the cardiac morphologic nding in this
1 patient.
A 20-year-old man died of gunshot wounds. There was no injury to the heart. At necropsy, the heart weighed 250 g. The left and right ventricular cavities were normal and no
grossly visible myocardial scars were seen. The left main and the right coronary arteries
arose normally from the left and right aortic sinuses, respectively (Figure1). The left main
coronary artery then divided into the left anterior descending and left circumex coronary
arteries, both of which thereafter coursed normally. In addition to the left main and the right
coronary ostia, a coronary ostial dimple was present in the wall of the aorta slightly above
the posterior aortic sinus (Figures 1 and 2). It measured 4mm in diameter and 3mm in
maximum depth.
Coronary artery ostia arise from the wall of the aortopulmonary trunk at the
time of embryonic division of the trunk.
consistently arise from the left and right aortic sinuses, with only minor variations
in their locations in most individuals. It has been suggested that the development
of the coronary ostial dimples in the wall of the aortopulmonary trunk require the
presence of a developing network of epicardial vessels in the heart.
vascular network, then, induces the formation of the coronary ostial dimples as it
approaches the aortopulmonary trunk. This hypothesis, however, would not explain
the presence of coronary ostial dimples in the opposite aortic sinus in patients with
“single coronary artery” and in the patient described here.
In addition to the heretofore described patient, at least 6 other cases of coronary
ostial dimples or buds have been reported (Table I).
from the present one in that the dimple was at a site where a coronary artery should
have arisen but did not. In other words, all 6 previously reported cases of coronary
dimple were in actuality examples of single coronary artery.
other hand, a coronary dimple was present at a site where a coronary ostium is not
normally present.
From the Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes of
Health, Building 10, Room 2N258, Bethesda, Maryland 20892. Dr. Robert’s current address is:
Baylor Cardiovascular Institute, Baylor University Medical Center, 3701 Junius Street, PO. Box
E010, Dallas, Texas 75246. Manuscript received December7, 1992; revised manuscript received
January29, 1993 and accepted February1.
264 DOI: 10.1201/9781003409342-45
1
It is not known why the coronary arteries
2
This epicardial
3–8
All 6 cases, however, differed
9
In our patient, on the
Case 983 Coronary ostIal dImple (In the posterIor aortIC sInus)
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Figure 1 Diagram (a) and photograph (b) of the longitudinally opened aortic valve
showing the positions of the 2 normal coronary ostia in the walls of the left (L) and
the right (R) aortic sinuses and the coronary ostial dimple above the posterior (P) aortic sinus. The horizontal broken line separates the sinus and the tubular portions of the
ascending aorta (sinotubular junction). The right and left coronary ostia are located
in the wall of the aorta slightly below and at this sinotubular junction, respectively.
The coronary ostial dimple is located slightly above the sinotubular junction.
Figure 2 Photomicrograph of longitudinal sections of aorta through the right coronary ostium (a), the coronary ostial dimple (b) and the left main coronary ostium
(c). The sections are taken as shown by the broken vertical lines through the coronary
ostia in Figure la. The aortic media (darker staining tissue) continues through the wall
of the ostial dimple. Movat stain ×13, reduced by 36%.
265
266
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Table I: Reported cases of coronary ostial dimple in the presence of “single coronary artery”
Patient Ref. Age (yr) & Sex Location of AP AMI SD CAD HW (g) LV Scar Cause of Death
“Single Ostium” Ostial Dimple
1 3 37M LAS RAS — — — — — — Infective endocarditis
2 4 68M LAS RAS — — — — — — Cancer
3 5 80F LAS RAS 0 0 0 + 340 0 Cancer
4 6 76F RAS LAS 0 0 0 0 370 0 Cancer
5 7 84F RAS LAS + + + + 530 + AMI
6 8 83F LAS PAS 0 0 + + 320 0 Ruptured CA aneurysm
AMI=acute myocardial infarct; AP=angina pectoris; CA=coronary artery; CAD=atherosclerotic coronary artery disease; F=female; HW=heart weight;
LAS=left aortic sinus; LV=left ventricular; M=male; PAS=posterior aortic sinus; RAS=right aortic sinus; SD = sudden death; — = information not available;
+=present; 0=absent.
Case reports In CardIology
Case 983 Coronary ostIal dImple (In the posterIor aortIC sInus)
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REFERENCES
1. Angelini P. Normal and anomalous coronary arteries: Denitions and classica-
tion. Am Heart J 1989;117:418–434.
2. Conte G, Pellegrini A. On the development of the coronary arteries in human
embryos, stage 14–19. Anat Embryol 1984;169:209–218.
3. Plaut A. Versorgung des Herzens durch nur eine Kranzarterie. Frankf Z Pathol
1922;27:84–90.
4. Ogden JA, Goodyer AVN. Patterns of distribution of the single coronary artery.
Yale J Biol Med 1970;43:11–21.
5. Smith JC. Review of single coronary artery with report of 2 cases. Circulation
1950;1:1168–1175.
6. Leivo IV, Laurila PK. Atresia of left coronary ostium and left main coronary
artery. Arch Pathol Lab Med 1987;111:1173–1175.
7. Vlodaver Z, Amplatz K, Burchell HB, Edwards JE. Single coronary ostium in
the aorta. In: Coronary Heart Disease: Clinical, Angiographic and Pathologic
Proles. New York: Springer-Verlag, 1976:189–216.
8. Causing WP, Shuster M, Pribor HC, Amboy P. Single coronary artery with rup-
tured coronary artery aneurysm. Arch Pathol 1967;83:419–421.
9. Shirani J, Roberts WC. Solitary coronary ostium in the aorta in the absence of other
major congenital cardiovascular anomalies. J Am Coll Cardiol 1993;21:137–143.
267
CASE REPORTS IN CARDIOLOGY
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Case 990 Sudden Death, Right Ventricular
Infarction, and Abnormal Right Ventricular
Intramural Coronary Arteries in Isolated
Congenital Valvular Pulmonic Stenosis
Jamshid Shirani, MD, Abarmard Maziar Zafari,
MD and William C. Roberts, MD*
Isolated congenital valvular pulmonic stenosis is a relatively common anomaly
of the heart. Fontana and Edwards
1
found pulmonic stenosis in 8 of 357 patients
(2%) with congenital cardiac disease studied at necropsy. In unoperated patients
with severe forms of this congenital anomaly, death is generally caused by progressive right ventricular failure.
adulthood is the rule.
fatal cases of isolated congenital valvular pulmonic stenosis, only 2 (aged 19 and
26years, both men) had died suddenly.
congenital cardiovascular disease (age range 1 to 21years) was caused by pulmonic
stenosis.
3
This report describes a patient with isolated pulmonic stenosis who died
1, 2
2
Sudden death is rare. In a review of 68 previously described
In those patients who survive infancy, survival into
2
Only 1 of 186 sudden deaths in patients with
suddenly, and at necropsy also was found to have right ventricular infarction and
abnormal intramural coronary arteries.
The patient, an 8-year-old black boy, had a precordial murmur since birth. He had normal growth and development, and was asymptomatic until age 5years when easy fatigability
was noted. Adiagnosis of “tetralogy of Fallot” was made by physical examination, but the
family refused further workup. Although his physical activities apparently were limited, his
only symptom was mild exertional dyspnea. He died suddenly at home. At necropsy, the
heart weighed 540 g (expected weight approximately 120). The pulmonic trunk was dilated
and its diameter was more than twice that of the ascending aorta (2.4 vs 1.1cm). The major
epicardial coronary arteries were normal. The pulmonic valve had a domeshaped structure
with a slightly eccentrically located orice that was 0.4cm
2
in area (Figure1). The right
atrial wall was markedly hypertrophied, and the cavity was dilated. The right ventricular
wall was markedly hypertrophied; the ventricular septum, which was thicker than the left
ventricular wall, was pushed toward the left ventricle, and no defect was present in the ventricular septum. Grossly visible scars were present in the moderator band, right ventricular
papillary muscles, and focally throughout the right ventricular free wall (Figures1 and 2).
Histologically, the right ventricular myocytes were larger than those of the ventricular septum and left ventricle, foci of interstitial and replacement brosis were present, and many
intramural coronary arteries in the right ventricular myocardium had thickened walls, especially in the areas of scarring (Figure2).
The present ndings indicate that sudden death may occur in patients with pul-
monic stenosis, despite few symptoms of cardiac dysfunction. Extensive scarring of
From the Pathology Branch, National Heart, Lung, and Blood Institute, National Institutes of
Health, Building 10, Room 2N258, Bethesda, Maryland 20892. Manuscript received January12,
1993; revised manuscript received and accepted March3, 1993.
* Current address: Baylor Cardiovascular Institute, Baylor University Medical Center, 3500
Gaston Avenue, Dallas, Texas 75246.
268 DOI: 10.1201/9781003409342-46
Case 990 sudden death, rIght VentrICular InFarCtIon
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Figure 1 The heart in the patient described. a, view of the stenotic pulmonic valve
from the pulmonary trunk (PT). b, view of the right (RA) and left (LA) atria, and the
great arteries after excision of the cephalad portion of both atria showing markedly
thickened and dilated right atrium, and the small-sized left atrium. c, photograph of
the cardiac ventricles after transverse incisions at approximately 1cm intervals from
base (upper left) to apex (lower right). The right ventricular (RV) wall is enormously
thickened, and scars (arrows) are visible, particularly in the moderator band. The
ventricular septum (VS) is concave toward the left ventricle (LV). AO=aorta.
the right ventricular wall can occur in this congenital cardiac anomaly and may be
related to abnormal intramural coronary arteries. Neither right ventricular scarring
nor abnormal right ventricular intramural coronary arteries were reported previously in patients with isolated valvular pulmonic stenosis.
269
Case reports In CardIology
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Figure 2 Photomicrographs of the ventricular myocardium in the patient described.
The nuclei of the right ventricular (RV) myocytes are darker and larger than those
of either the ventricular septum (VS) or left ventricular (LV) wall. Hematoxylineosin stains each ×400; reduced by 50%. Bottom left, photomicrograph showing a large,
thickened intramural coronary artery in the RV wall. Movat stain ×100; reduced by
50%. Bottom right, photomicrograph showing considerable interstitial brosis in the RV
wall. Movat stain ×100; reduced by 50%.
REFERENCES
1. Fontana RS, Edwards JE. Congenital Cardiac Disease: A Review of 357 Cases
Studied Pathologically. Philadelphia and London: W.B. Saunders, 1962:102–105.
2. Greene DG, Baldwin ED, Baldwin JS, Himmelstein A, Roh CE, Coumand A. Pure
congenital pulmonary stenosis and idiopathic congenital dilatation of the pulmonary artery. Am J Med 1949;6:24–40.
3. Lambert EC, Menon VA, Wagner HR, Vlad P. Sudden unexpected death from car-
diovascular disease in children. Am J Cardiol 1974;34:89–96.
270
Case 1545 “repaIred” tetralogy oF Fallot mImICkIng rIght CardIomyopathy
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Case 1545 “Repaired” Tetralogy of Fallot
Mimicking Arrhythmogenic Right Ventricular
Cardiomyopathy (Another Phenocopy)
Betsy Ann George, MD
Johannes Jacob Kuiper, MD
a,d
, Jong Mi Ko, BAd, Forrester Dubus Lensing, MDb,
a,d
and William Clifford Roberts, MD
a,c,d,
*
Described is a 41-year-old man who at age 6 had partial resection of an
obstructed right ventricular outow tract with insertion of a patch and
closure of a ventricular septal defect (tetralogy of Fallot). At age 41, cardiac
transplantation was performed because of right ventricular outow patch
aneurysm, numerous episodes of ventricular tachycardia, and chronic heart
failure, all features of the familial form of arrhythmogenic right ventricular
cardiomyopathy (ARVC). Additionally, the patient had bundle branch block
and epsilon waves on electrocardiogram, other features of ARVC. The case is
described to introduce the concept of acquired ARVC, because the patient had
many of the clinically recognized features of familial ARVC.
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by
localized thinning of the right ventricular (RV) wall with the localized portion consisting mainly of brofatty tissue.
to aneurysm formation.
2
ARVC also is characterized by the presence of epsilon
1–3
The thinning of the RV wall commonly leads
waves, bundle branch block, late potentials, and ventricular tachyarrhythmias on
the electrocardiogram. ARVC has been recognized exclusively as a familial disease
with autosomal dominant inheritance.
1, 2
In this report, we introduce the concept of
acquired ARVC in a patient who was operatively treated for tetralogy of Fallot and
later developed features of ARVC.
CASE DESCRIPTION
A 41-year-old man, born on October 10, 1969, had a right Blalock-Taussig shunt
placed in 1974, at 4years of age, for tetralogy of Fallot. In 1976, at age 6, he underwent
widening of the RV outow tract using a Dacron patch (4.5 × 3cm) and closure of
a large (2 × 3cm) subaortic ventricular septal defect. Preoperatively, the peak systolic pressure gradient between the right ventricle and pulmonary trunk had been
85mm Hg. The patch extended through the nonobstructive pulmonary valve, and 2
of its 3 cusps were excised. The Blalock-Taussig shunt was ligated. Immediately after
operation, the peak systolic RV pressure was 70mm Hg and that in the pulmonary
trunk 35mm Hg. The peak systolic left ventricular pressure was 100mm Hg.
The patient thereafter was asymptomatic until 26years of age, when he had his
rst episode of ventricular tachycardia (VT). Transthoracic echocardiogram performed at age 26 revealed a peak systolic pressure gradient between the right ventricle and pulmonary trunk of 25mm Hg, marked RV dilatation, a mildly dilated
Departments of aInternal Medicine (Division of Cardiology), bRadiology, and cPathology and
d
The Baylor Heart and Vascular Institute, Baylor University Medical Center, Dallas, Texas.
Manuscript received January27, 2011; revised manuscript received and accepted March7, 2011.
* Corresponding author: Tel: 214-820-7911; fax: 214-820-7533.
E-mail address: wc.roberts@baylorhealth.edu (W.C. Roberts).
DOI: 10.1201/9781003409342-47
271
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